The relationship between stress, anxiety, and depression levels and pseudobulbar affect in patients with multiple sclerosis.

BACKGROUND: Pseudobulbar affect (PBA) has recently been added to the list of mental disorders commonly observed in patients with Multiple Sclerosis (MS). The emotional changes experienced by MS patients may be associated with other mental problems and adversely affect disease prognosis. However, there are limited studies in the international and national literature on this subject. AIM: This study aimed to evaluate the relationship between PBA and stress, anxiety, and depression levels in patients with MS. DESIGN: This is a descriptive, relational, and cross-sectional study. METHODS: A total of 442 MS patients followed in the neurology outpatient clinic of a hospital and registered with the MS Society in Turkey participated in this study. Data were collected using a personal information form, the Turkish version of the Center for Neurologic Study-Lability Scale (CNS-LS), and the Depression Anxiety Stress Scale (DASS). RESULTS: In this study, 63.3% of the patients had PBA, 21% had extremely severe depression, 41.6% had extremely severe anxiety, and 24.9% had severe stress. Correlation analysis revealed weak positive correlations between CNS-LS total score and DASS subscale and total scores. Depression, anxiety, and stress levels accounted for 22.5% of the variation in CNS-LS total score. CONCLUSION: The results of this study indicate that a majority of MS patients in Turkey experience PBA, depression, anxiety, and stress, and that PBA is positively associated with depression, anxiety, and stress. IMPACT STATEMENT: This study, indicates that should be increased consultation-liaison psychiatry services in Turkey, and MS patients should benefit from comprehensive psychiatric services.

[Acupoint selection rules of acupuncture for pseudobulbar palsy dysphagia].

OBJECTIVE: To analyze the acupoint selection rules of acupuncture for pseudobulbar palsy dysphagia using data mining technology. METHODS: The literature of acupuncture for pseudobulbar palsy dysphagia published from January 1, 1990 to May 1, 2021 was retrieved from CNKI, SinoMed, Wanfang, VIP, and PubMed databases. Acupuncture prescription database was established. The frequency of acupoint selection was analyzed by Microsoft Excel 2016; Apriori algorithm was used to analyze the association rules and draw the high-frequency acupoint co-occurrence network diagram; SPSS21.0 was used to perform clustering analysis. RESULTS: A total of 87 literature was included, involving 89 acupuncture prescriptions and 71 acupoints. Fengchi (GB 20) was the most frequently-used acupoint; the commonly-selected meridians were gallbladder meridian, conception vessel, governor vessel and stomach meridian; the acupoints located at the neck were the most frequently-used acupoints; the crossing points were commonly selected among the special acupoints. The most commonly-used acupoint combination was Jinjin (EX-HN 12) plus Yuye (EX-HN 13). CONCLUSION: The modern acupuncture for pseudobulbar palsy dysphagia usually selects local acupoints, especially the neck acupoints such as Fengchi (GB 20) and Lianquan (CV 23). The acupoints in the front and back are concurrently selected with needles towards the disease location.

Effect of acupuncture treatment on dysphagia caused by pseudobulbar paralysis after stroke: a systematic review and meta-analysis.

BACKGROUND: The efficacy of acupuncture in the treatment of dysphagia caused by pseudobulbar paralysis after stroke is lack of evidence-based medicine. Our objective was to synthesize the efficacy of acupuncture in treating dysphagia caused by pseudobulbar paralysis after stroke. METHODS: A comprehensive literature search was performed in 9 databases [PubMed, Web of Science, Embase, Cochrane, Chinese BioMedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), WanFang Data, Chinese Science and Technology Periodicals database (VIP), and Open Grey online database] to screen eligible randomized controlled studies that evaluated the effect of acupuncture in dysphagia caused by pseudobulbar paralysis after stroke. The search time limit is from establishing the database to October 1, 2020. The random-effects model was used to calculate the significant effect size. RESULTS: A total of 7 studies comprising 637 participants were included in our meta-analysis. The results showed that compared with rehabilitation, acupuncture had a significant effect on improving dysphagia caused by pseudobulbar paralysis after stroke [the significant effective size: risk ratio (RR)sig =1.51; 95% confidence interval (CI): 1.30-1.75; I2=0%]. In the subgroup analyses, the RRsig of acupuncture + rehabilitation vs. rehabilitation was 1.56 (95% CI: 1.30-1.87; I2=0%), and the RRsig of acupuncture vs. rehabilitation was 1.38 (95% CI: 1.08-1.76; I2=0.8%). DISCUSSION: Acupuncture can be used as an effective treatment for dysphagia caused by pseudobulbar paralysis after stroke. Acupuncture combined with rehabilitation therapy has better effects.

Occipital Interhemispheric Transtentorial Approach for a Pineal Region Meningioma with Pseudobulbar Palsy.

Meningiomas represent 8%-10% of all pineal region (PR) tumors. When they arise from the falx, tentorium, or tentorial incisura, they are not always considered a true PR tumor, as they do not originate from it but instead only grow toward the region. The true meningioma of PR must be originated from the arachnoid envelope of the pineal gland or from the 2 leaflets of the velum interpositum. In both conditions there are no dural attachments.1,2 Occipital interhemispheric transtentorial and supracerebellar infratentorial are the 2 main approaches for tumors within this region. Aside from the surgeon's preference, the position of the venous system and the direction of the tumor growth guide the approach choice. Endoscope assistance can help reach areas unable to be visualized under the microscope.3-5 We report the case of a 37-year-old female with a large PR meningioma (velum interpositum) presenting with intense dysphagia, dysphonia, and bilateral tongue palsy. Given the affected bilateral upper motor tracts associated with bulbar symptoms, a diagnosis of pseudobulbar palsy was considered.6,7 Preoperative imaging also showed compression of the deep venous system. The patient underwent a total resection of the tumor via an occipital interhemispheric transtentorial approach and exhibited a dramatic recovery of neurologic symptoms after the surgery (Video 1). Postoperative venogram showed restoration of the usual deep venous system pattern, which may be associated with significant neurologic improvement. Careful management of the deep veins is mandatory during the resection of PR meningiomas. The venous system improvement after the surgery may be associated with the dramatic recovery seen in this unique case. The patient consented to publication of her images.

Rare case of pseudobulbar palsy: subopercular syndrome.

We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which deteriorated rapidly to anarthria and aphonia within a few days. MRI scan of the head showed acute infarct in right internal capsule and an old infarct in the left corona radiata while the rest of the investigations were normal. Based on these findings, diagnosis was thought to be subopercular syndrome. He recovered significantly in a few weeks' time.

Isolated acute pseudobulbar palsy with infarction of artery of percheron: case report and literature review.

INTRODUCTION: Pseudobulbar palsy (PBP) is characterized by supranuclear lesions in the corticobulbar pathway. Neoplasia, inflammatory, demyelinating, and stroke are possible etiologies of this disorder. CASE REPORT: We report an elderly female who presented with dysarthria. She was dysarthric with a hypernasal voice, no apraxia or aphasia was observed. Tongue movements were slow with limited amplitude. Her soft palate dropped bilaterally; gag reflex was present. Also, she reported swallowing difficulty and choking with her saliva. Bilateral vertical and horizontal gaze were intact to either voluntary or oculocephalic movements. A cranial CT scan was suggestive of artery of Percheron (AOP) infarction. Brain magnetic resonance imaging showed hypersignal on diffusion-weighted and T2-weighted images and hyposignal on apparent diffusion coefficient in both thalami. CT angiography scan revealed an AOP originating from the left posterior cerebral artery. The swallowing study with a videofluoroscopic demonstrated oral and pharyngeal phases with severe dysfunction. CONCLUSION: To the authors' knowledge, there are two cases of individuals with artery of Percheron infarction who developed PBP associated with other clinical syndromes. Still, isolated PBP following infarction of Percheron's artery was not reported. We hypothesized that the PBP may have occurred because of the existence of vascular territory variations in the perforating arteries that arise from the AOP.

Claustrum hyperintensity: a rare radiological correlate in Niemann-Pick disease.

A 5-year-old male child of consanguineous parentage, without any adverse perinatal history, presented with progressive cognitive regression predominantly in the language and attention domains, for 2 years. He had simultaneous pyramidal and extrapyramidal involvement, frequent generalised tonic-clonic seizures and recurrent respiratory tract infections. Examination was significant for vertical supranuclear gaze palsy, coarse facial features and splenomegaly. Given the clinical features, in the background of consanguinity and mother's history of spontaneous pregnancy losses, inborn errors of metabolism were suspected. Following relevant investigations including tailored genetic study, Niemann-Pick disease type C (NPC) was diagnosed. Interestingly, MRI brain showed bilateral T2/fluid-attenuated inversion recovery claustrum hyperintensities, which are more commonly associated with autoimmune encephalitis and febrile infection-related epilepsy syndrome and not reported previously in NPC. Additionally, language regression as a presenting manifestation in NPC as opposed to classical dysarthria makes this case truly unique.

Dramatic Response to Pramipexole in Delayed-Onset Parkinsonism from Osmotic Demyelinating Syndrome.

Background: Delayed parkinsonism and dystonia are recognized phenomena in osmotic demyelinating syndrome (ODS). Dopamine receptor agonists and levodopa have been reported to benefit select patients. Case report: We report a patient with ODS with severe pseudobulbar deficits, parkinsonism and dystonia, poorly responsive to levodopa, who experienced a remarkable improvement with pramipexole. Discussion: A marked response to pramipexole with lack of response to levodopa suggests a pre-synaptic source for his deficits coupled with injuries to non-nigral compensatory structures. Highlights: This case highlights a dramatic response of osmotic demyelination-induced parkinsonism/dystonia to pramipexole. A lack of response to levodopa suggests deficits in the pre-synaptic nigral as well as non-nigral compensatory structures.

[Fire needling on dysphagia due to pseudobulbar palsy after stroke: a randomized controlled trial].

OBJECTIVE: To observe the clinical effect of fire needling on dysphagia due to pseudobulbar paralysis after stroke and to compare the difference in clinical effect between fire needling and swallowing function rehabilitation training. METHODS: A total of 76 patients with dysphagia due to pseudobulbar paralysis after stroke were randomly divided into an observation group and a control group, 38 cases in each group (1 case dropped out in the control group). The both groups were based on conventional western medication treatment. Fire needle pricking was exerted at Lianquan (CV 23), Fengchi (GB 20), Wangu (GB 12), Shuigou (GV 26), Neiguan (PC 6) and Zusanli (ST 36) without needle retaining every other day in the observation group. The control group was treated with the swallowing function rehabilitation training. In both groups, treatment for 2 weeks was as one course and 2 courses of treatment with 2-day interval were required. After treatment, swallowing scores of Fujishima Ichiro and swallow quality of life questionnaire (SWAL-QOL) scores were observed in both groups, and the clinical effect was compared. Follow-up of swallowing scores of Fujishima Ichiro in 4 weeks after treatment was completed to evaluate the clinical effect. RESULTS: The clinical effective rates after treatment and follow-up were 92.1% (35/38) and 94.7% (36/38) in the observation group, higher than 75.7% (28/37) and 83.8% (31/37) in the control group (P<0.05). After treatment, the swallowing scores of Fujishima Ichiro and SWAL-QOL scores were increased in the two groups (P<0.05), and those in the observation group were higher than the control group (P<0.05). The swallowing scores of Fujishima Ichiro were increased during follow-up in the two groups (P<0.05). CONCLUSION: Fire needling has a better effect than conventional rehabilitation training in the treatment of dysphagia due to pseudobulbar paralysis after stroke, which can obviously improve the swallowing function and quality of life in patients with dysphagia.

Neuropsychiatric profile of patients with craniocervical dystonia: A case-control study.

OBJECTIVE: Depression, anxiety, and obsessive-compulsive disorder have been widely reported in patients with dystonia. On the other hand, cognitive impairment, frontal lobe function, impulsiveness and pseudobulbar affect are less studied. The objective of the study is to assess these neuropsychiatric symptoms along with the quality of life of subjects with craniocervical dystonia. PATIENTS AND METHODS: A cross-sectional study was carried out in patients with craniocervical dystonia. Sex- and age-matched healthy controls were included. Neuropsychiatric assessment included the Montreal Cognitive Assessment (MoCA), Frontal Assessment Battery (FAB), Barrat Impulsiveness Scale (BIS-11), Center for Neurologic Study-Lability Scale (CNS-LS), Anxiety Rating Scale (HAM-A), Hamilton Depression Rating Scale (HAM-D), and the 12-item Short Form Health Survey (SF-12). RESULTS: A total of 44 patients with craniocervical dystonia and 44 controls were included. The mean age was 57 ± 13.7 years. Depression (56.1 % vs 9.1 %, p < 0.001), anxiety (56.8 % vs 6.8 %, p < 0.001), and pseudobulbar affect (31.8 % vs 9.1 %, p = 0.02) were more common in the dystonia group in comparison to controls. No difference between groups was found in impulsiveness (p = 0.65), MoCA score (p = 0.14) or executive dysfunction (p = 0.42). Quality of life was worst in the dystonia group with 90.9 % (p = 0.03) and 61.4 % (p < 0.001) of the subjects scoring under average in the Physical Composite Score (PCS) and Mental Composite Score (MCS) of the SF-12. CONCLUSION: MoCA scores ≤18, pseudobulbar affect, depression and anxiety are more prevalent in subjects with craniocervical dystonia in comparison to sex- and age-matched healthy controls. Regarding quality of life, MCS is more affected that the PCS in subjects with dystonia.

Thoracic Dural Arteriovenous Fistula Presenting with Isolated Pseudobulbar Palsy Mimicking Brainstem Lesion.

BACKGROUND: Spinal dural arteriovenous fistulas (DAVFs) are usually associated with neurologic dysfunction adjacent to the shunt point; however, the symptoms are uncommon far from the site of the fistula. To our knowledge, this is the first report of a patient with rapidly progressive isolated pseudobulbar palsy because of thoracic DAVF. CASE DESCRIPTION: We report a patient with thoracic DAVF presenting with remote symptoms of brainstem congestion. The patient was a 36-year-old man who presented with a sudden history of vomiting, dysphagia, and flaccid weakness in the 4 limbs. Intracranial magnetic resonance (MR) imaging at a local hospital demonstrated T2 signal hyperintensity within the medulla, and he was referred to our hospital for a suspected brainstem lesion. However, cervical MR imaging revealed a dilated and tortuous perimedullary venous plexus, and spinal angiography revealed DAVF in T5-6 with a feeding artery from the intercostal artery. After obliteration of the fistula, the progression of the disease was stopped and the symptoms improved. CONCLUSIONS: Although rare, thoracic DAVFs may present symptoms resembling brainstem infarction. Prompt surgical intervention is necessary for patients with thoracic DAVF presenting with rapidly progressive pseudobulbar palsy.

Management of the pseudobulbar affect (PBA) in Kabuki syndrome combined dextromethorphan-fluoxetine treatment as an alternative to dextromethorphan/quinidine

A case report of a patient with pseudo bulbar affect previous treatments included haloperidol (10mg), Inosina pranobex (600mg), clozapine (600mg), olanzapine (20mg), carbamazepine (200mg), paroxetine (20mg), phenobarbital (100mg) and topiramate (50mg), all suspended at August 2016, with current use of quetiapine (700mg) Chlorpromazine (600mg) (+ 200mg on demand of aggression), clonazepam (4 mg), valproate 2500 mg, propranolol (40mg). that was successful treated with off label treatment (dextromethorphan plus quinidine). Previous Brief Psychiatric Rating Scale and Clinical Global Impression-Improvement was applied after and before treatment with dextromethorphan (20mg) plus fluoxetine (20 mg, further increased to 40 mg). Previous Brief Psychiatric Rating Scale BPRS score 56 points and Clinical Global Impression-Severity (CGI-S) Score was 6 (severely ill). The addition of dextromethorphan (20mg) and fluoxetine (20 mg, further increased to 40 mg), allowed clear improvement of pathological crying and outbursts, with BPRS decrease of 8 points and Clinical Global Impression-Improvement (CGI-I) 2 (much improved) ­ especially pertaining to PBA related symptoms and aggressive behavior. There were no noticeable side-effects. This case report shown an interesting clinical response. It's could be a great alternative in treatment of pseudobulbar affect symptoms. Even though an only case and a great clinical study be necessary. (AU)

The psychopharmacology of pseudobulbar affect.

Pseudobulbar affect (PBA) is characterized by uncontrollable emotional episodes disconnected or disproportionate with mood, in association with an array of neurologic conditions. PBA is associated with disruption of descending control of brainstem motor circuitry and dysregulation of serotonergic and glutamatergic function. PBA has been historically under recognized, though advances resulting in more specific diagnostic criteria, validated rating scales, and an approved pharmacotherapy offer opportunities for improved treatment outcomes.

[Foix-Chavany-Marie Syndrome: A Clinical Overview].

Foix-Chavany-Marie syndrome, which has been reported since the 19th century, is a cortical type of pseudobulbar palsy. Although previously most cases were due to acute recurrent stroke, recent reports have indicated that there are various causes, including infectious, developmental, epileptic and degenerative processes and various clinical courses, which may be transient or slowly progressive. Moreover, modern imaging techniques have revealed the significance of the injury of the white matter tract, such as the frontal aslant tract, rather than classical lesions of bilateral motor cortices. Considering both the findings of the bedside examination of patients with this "old" syndrome and of "new" imaging studies performed at appropriate times is expected to contribute to our understanding of the neural underpinnings of this syndrome.

[An Autopsy Case of Amiotrophic Lateral Sclerosis Characterized by Upper Motor Neuron Degeneration and Progressive Pseudobulbar Palsy].

This is a case report of autopsy findings for a male Japanese patient who presented with progressive gait disturbance and dysarthria. Neurological examination at the age of 61 years revealed pseudobulbar palsy and upper motor neuron disorder. The patient appeared unaware of his illness. Electrophysiological examination showed lower neuron damage. The patient was diagnosed with amyotrophic lateral sclerosis characterized by lower motor neuron damage. He died of pneumonia 2 years and 3 months after diagnosis. Neuropathological examination demonstrated severe degeneration of the upper neurons and mild degeneration of the lower neurons. Immunohistological examination indicated transactivation-responsive DNA-binding protein-43-positive pathology in the frontal and temporal lobes, amygdala, corpus striatum, and spinal cord. We believe the neuropathological findings correlate well with the clinical features. Furthermore, we also discuss the lesions involved in the patient's dementia. (Received March 28, 2017; Accepted September 19, 2018; Published November 1, 2018).

Assessment of Use of Combined Dextromethorphan and Quinidine in Patients With Dementia or Parkinson Disease After US Food and Drug Administration Approval for Pseudobulbar Affect.

Importance: In 2010, the US Food and Drug Administration (FDA) approved a combination of dextromethorphan hydrobromide and quinidine sulfate for the treatment of pseudobulbar affect after studies in patients with amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS). This medication, however, may be commonly prescribed in patients with dementia and/or Parkinson disease (PD). Objective: To investigate the prescribing patterns of dextromethorphan-quinidine, including trends in associated costs. Design, Setting, and Participants: This population-based cohort study of patients prescribed dextromethorphan-quinidine used data from 2 commercial insurance databases, Optum Clinformatics Data Mart and Truven Health MarketScan. The Medicare Part D Prescription Drug Program data set was used to evaluate numbers of prescriptions and total reported spending by the Centers for Medicare & Medicaid Services. Patients were included if they were prescribed dextromethorphan-quinidine from October 29, 2010, when the drug was approved, through March 1, 2017, for Optum and December 31, 2015, for Truven. Data were analyzed from December 1, 2017, through August 1, 2018. Main Outcomes and Measures: The proportion of patients prescribed dextromethorphan-quinidine with a diagnosis of MS, ALS, or dementia and/or PD, as well as the number of patients with a history of heart failure (a contraindication for the drug). Results: In the commercial health care databases, 12 858 patients filled a prescription for dextromethorphan-quinidine during the study period. Mean (SD) age was 66.0 (18.5) years, 66.7% were women, and 13.3% had a history of heart failure. Combining results from both databases, few patients had a diagnosis of MS (8.4%) or ALS (6.8%); most (57.0%) had a diagnosis of dementia and/or PD. In the Medicare Part D database, the number of patients prescribed dextromethorphan-quinidine increased 15.3-fold, from 3296 in 2011 to 50 402 in 2016. Reported spending by Centers for Medicare & Medicaid Services on this medication increased from $3.9 million in 2011 to $200.4 million in 2016. Conclusions and Relevance: Despite approval by the FDA for pseudobulbar affect based on studies of patients with ALS or MS, dextromethorphan-quinidine appears to be primarily prescribed for patients with dementia and/or PD.

Pseudobulbar Affect in Survivors of Extreme Prematurity With Cerebellar Injury: Support for the Cerebellar Link in Pathologic Laughter and Crying.

Pseudobulbar affect, that is, pathologic laughter and crying is being increasingly recognized in adults and is seen in association with a number of diseases like Parkinson disease, dementia, traumatic encephalopathy, and others, but has not previously been described in children with cerebral palsy. The condition pseudobulbar affect may be due to lesions in (or degeneration of) the cerebro-ponto-cerebellar pathways. Here we report 2 children with cerebral palsy who have structural cerebellar injury because of their being born extremely premature who have pathologic crying and probably laughter.